Idiopathic angioedema is a term applied to recurrent episodes of angioedema of unknown etiology. The following is a case report of idiopathic recurrent isolated orbital angioedema with exophthalmos which responds to prolonged courses of oral corticosteroids.
Methods:
A 67 year old Caucasian female with aspirin exacerbated respiratory disease (AERD) sought treatment for an acute, progressive painless left eye swelling with exophthalmos without visual deficits or urticaria. High dose corticosteroids were initiated followed by a low maintenance dose. The swelling subsided after one year of corticosteroid therapy.
Ten years later, orbital swelling with exophthalmos returned in the same eye. No medications, such as aspirin(1) or non steroidal anti-inflammatory drugs(2), were associated with the swelling. A CT of the orbits revealed an isolated proptosis with swelling of the medial and inferior rectus muscles and mild hypertrophy and swelling of the left lacrimal gland. A complete history and physical examination were negative. The family history likewise was negative.
Results:
High-dose systemic glucocorticoid therapy was initiated. Symptoms resolved after 1 month of tapered corticosteroid therapy, however, swelling reoccurred in the orbit within one week. Low dose maintenance corticosteroids were reinitiated with resolution of the orbital swelling. Work-up for acquired C1 esterase deficiency is negative.
Conclusions:
An atypical case of recurrent idiopathic isolated orbital angioedema with exophthalmos in a patient with AERD and no triggering factor, systemic findings and a negative evaluation is presented.
1. Quiralte J. Aspirin-induced isolated periorbital angioedema. Ann Allergy Asthma Immunol. 1998;81(5 Pt 1):459.
2. Marshall LL. Angioedema associated with aspirin and rofecoxib. Ann Pharmacother. 2005;39(5):944-8.